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Arrhythmogenic Right Ventricular Cardiomyopathy

26 bytes added, 22:59, 27 January 2010
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[[Image:epsilon_wave.png|thumb|ECG with an epsilon wave in V1]]
[[Image:arvdhart.png|thumb| A section throught throughout the heart of a an ARVC patient. (A) Transmural fatty replacement of the right ventricular free wall. (B) Myocardial atrophy is confined to the right ventricle and substantially spares the interventricular septum as well as the left ventricular free wall. <cite>Corrado</cite> Reproduced with permission from BMJ Publishing Group Ltd. ]]'''Arrythmogenic Arrhytmogenic Right Ventricular Cardiomyopathy''', (ARVC, or ARVD: Arrythmogenic Arrhythmogenic Right Ventricular Disease) is characterized by fatty replacement and fibrosis of the heart. Especially the right ventricle apex and outflow tract are involved. However the left ventricle can be affected to.<cite>Corr</cite>
As a result of the fatty replacement and fibrosis, ventricular arrhythmias are common in this disease and can lead to palpitations, syncope and sudden death. At older age more advanced ages right ventricular pump failure can occur.
The diagnosis is based on major and minor criteria, as published by the European Society of Cardiology.<cite>McKenna1994</cite>
ARVC is a progressive disease. The '''incidence''' is estimated to be 1:3.000-1:10.000. Manifestations is are usually seen during teenagethe teens. Although the diagnosis is more often made in athletes, sports are not thought to have a causative relationship with the disease. ARVD can occur in families; more than 9 different chromosomal defects have been described, most often with autosomal dominant inheritance.
One unique form of ARVD, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance.
'''Diagnosis''' ARVC is a difficult diagnosis to make. Therefore, the European Society of Cardiology has created a list of diagnositc diagnostic criteria for the diagnosis of ARVC<cite>#McKenna1994</cite> (see table).
{| class="wikitable" width="400px"
!Major diagnostic criteria for Aritmogenic Arrhythmogenic Right Ventricular Cardiomyopathy<cite>McKenna1994</cite>
|-
|<ul><li>Familial disease confirmed at necroscopy or surgery</li>
<li>Severe dilatation and reduciton reduction of right ventricular ejection fraction with no (or only mild) LV impairment</li><li>Localised irhgt Localized right ventricular aneurysms (akinetic or diskinetic areas with diastolic bulging)</li>
<li>Severe segmental dilataion of the right ventricle</li>
<li>Fibrofatty replacement of myocardium on endomyocardial biopsy</li>
|-
|<ul>
<li>(major) Epsilon wave or localised localized prolongation (>110ms) of the QRS complex in right precordial leads (V1-V3)</li>
<li>(minor) Inverted T waves in right precordial leads (V2 and V3) (people aged more than 12 yr; in absence of [[RBBB]]</li>
<li>(minor) [[Late potentials]] ([[SAECG|signal averaged ECG]])</li>
<li>(minor) Left bundle branch block type [[Ventricular Tachycardia|ventricular tachycardia]] (sutained sustained and non-sustained) (ECG, [[Holter]], [[Exercise Testing|exercise testing]]</li>
<li>(minor) Frequent [[Ventricular Premature Beats|ventricular extrasystoles]] (more than 1000/24h) ([[Holter]])</li>
</ul>
'''Treatment''' focuses on avoiding complications.<cite>ACC2006</cite>
*Medication:
**anti-arrithmicsarrhythmics: Sotalol better than Amiodarone.
**ACE-inhibitors to prevent cardiac remodelling
*[[ICD]] implantation is recommended for the prevention of sudden cardiac death in patients with ARVC with documented sustained VT or VF who are receiving chronic optimal medical therapy.
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